Outcomes Following Endoscopic Resection of Craniopharyngiomas in the Pediatric Population

Background: Craniopharyngiomas have traditionally been treated via open, transcranial approaches. More recently, minimally invasive endoscopic trans-sphenoidal approaches have been increasingly utilized. There is not yet a consensus in the literature whether one approach is superior to the other. Specifically regarding the pediatric population, there is a paucity of case series in our literature regarding long-term outcome measures of this newer technique with respect to the management of craniopharyngiomas. We report our institutional experience.

Objective: To assess the outcomes of endoscopic trans-sphenoidal resection of pediatric craniopharyngiomas with special attention to symptom resolution, disease recurrence, extent of resection, visual outcomes, endocrine outcomes, and CSF leak.

Methods: A retrospective review of patients undergoing endoscopic trans-sphenoidal resection of craniopharyngiomas at a tertiary pediatric hospital between 1995 and 2016 was performed. Only pediatric cases (< 18 years of age) of craniopharyngioma confirmed by pathology were included. Patient demographic data including total follow-up time, tumor size, and tumor location were reviewed. Outcome measures included presenting symptoms, post-operative symptoms, visual outcomes, endocrine outcomes, disease recurrence, extent of recurrence, and major complications defined by visual compromise, vascular injury or death.

Results: A total of 16 pediatric patients (mean age = 11.0) with trans-sphenoidal, endoscopic resection of craniopharyngioma were included. The median follow-up time after resection was 4.14 years. Median maximal tumor diameter was 2.7cm, ranging from 1.8cm - 9.0cm. The majority of the tumors had suprasellar (93.8%) and intrasellar (68.8%) components, however, one was retroclival and one was infrasellar/extracranial. Six patients had undergone multiple prior neurosurgical interventions. All but one patient (93.8%) had attempted gross-total resection. The most common presenting symptoms were vision changes (75.0%) and signs of increased intracranial pressure (56.3%). The majority of patients had resolved their presenting symptoms (86.6%) at their first post-op visit. Vision improved or remained normal in 57.1% of patients, and none reported worsening vision after surgery. New incidence of panhypopituitarism or diabetes insipidus (DI) developed in 62.5% and 43.8% of patients following surgery, respectively. The post-operative CSF leak was 18.7%. These patients were highly correlated with a significantly larger tumor size (average size of 4.2cm), and prior neurosurgical intervention. Five previous procedures had been performed among the three patients with CSF leak before they reached our institution. One of these patients had previously undergone 3 endoscopic resections and a burr hole aspiration at an outside hospital. One patient died from intraventricular hemorrhage post-operatively, and this was also associated with larger tumor size. The major complication rate was 6.3%. Disease recurrence was seen in two patients (12.5%). As expected, one of these patients was the only sub-total resection in this case series.

Conclusion: Minimally invasive endoscopic trans-sphenoidal resection for craniopharyngioma can achieve high rates of total-resection with low rates of disease recurrence. The goal of total resection must be balanced with potential complications. Complications following endoscopic resection are more likely in patients with larger tumor size and previous resection attempts.